Dejerine-Thomas Syndrome

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Classification according to ICD-10
G90.3 Multiple system atrophy
G23.8 Other specified degenerative diseases of the basal ganglia
- Dejerine-Thomas syndrome
ICD-10 online (WHO version 2019)

The Dejerine-Thomas syndrome ( synonyma olivopontocerebellar atrophy , bridge atrophy type Sparrow) in its sporadic form ( SOPCA ) comprises a combination of atrophy of the system of the bridge foot with coordination disorders from the cerebellum such as ataxia , speech disorders ( dysarthria ) and tremors ( tremor ).

It was first described in 1900 by the French neurologist Joseph Jules Dejerine and his student and colleague André Antoine Henri Thomas .

The syndrome should not be confused with Thomas syndrome , a very rare malformation syndrome .

diagnosis

Clinically, there is a chronically progressive ataxia with increasing cerebellar atrophy and balance and walking disorders, involuntary movements, speech disorders, nystagmus and subtle tremors.

literature

  • A. Fickler: Clinical and pathological-anatomical contributions to the diseases of the cerebellum. In: German journal for neurology. 1911, 41, pp. 306-375.
  • H. Kulawik, E. Roitzsch, H. Barz: [The clinical picture of olivo-ponto-cerebellar atrophy (Dejerine-Thomas)]. In: Psychiatry, Neurology, and Medical Psychology. Volume 24, Number 3, March 1972, pp. 119-127. ISSN  0033-2739 . PMID 5034933 .
  • LR Caplan: Clinical features of sporadic (Dejerine-Thomas) olivopontocerebellar atrophy. In: Advances in neurology. Volume 41, 1984, pp. 217-224, ISSN  0091-3952 . PMID 6496227 .

Individual evidence

  1. ^ B. Leiber: The clinical syndromes. Syndromes, sequences and symptom complexes. Edited by G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger, 7th edition. Urban & Schwarzenberg 1990, ISBN 3-541-01727-9 .
  2. ^ JJ Dejerine, A. Thomas: L'átrophie olivo-ponto-cérébelleuse. In: Nouvelle iconographie de la Salpêtrière. 1900, 13, p. 330.
  3. ↑ Who named it

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